{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T05:21:08Z","timestamp":1790659268664,"version":"4.1.0"},"reference-count":67,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2018,7,12]],"date-time":"2018-07-12T00:00:00Z","timestamp":1531353600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"},{"start":{"date-parts":[[2018,8,1]],"date-time":"2018-08-01T00:00:00Z","timestamp":1533081600000},"content-version":"vor","delay-in-days":20,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100012443","name":"Maryland Stem Cell Research Fund","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100012443","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008510","name":"University of Maryland","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008510","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["asbmr.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2018,11,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>\u2003<\/jats:title><jats:p>Patients with Hutchinson-Gilford progeria syndrome (HGPS) have low bone mass and an atypical skeletal geometry that manifests in a high risk of fractures. Using both in vitro and in vivo models of HGPS, we demonstrate that defects in the canonical WNT\/\u03b2-catenin pathway, seemingly at the level of the efficiency of nuclear import of \u03b2-catenin, impair osteoblast differentiation and that restoring \u03b2-catenin activity rescues osteoblast differentiation and significantly improves bone mass. Specifically, we show that HGPS patient-derived iPSCs display defects in osteoblast differentiation, characterized by a decreased alkaline phosphatase activity and mineralizing capacity. We demonstrate that the canonical WNT\/\u03b2-catenin pathway, a major signaling cascade involved in skeletal homeostasis, is impaired by progerin, causing a reduction in the active \u03b2-catenin in the nucleus and thus decreased transcriptional activity, and its reciprocal cytoplasmic accumulation. Blocking farnesylation of progerin restores active \u03b2-catenin accumulation in the nucleus, increasing signaling, and ameliorates the defective osteogenesis. Moreover, in vivo analysis of the Zmpste24-\/- HGPS mouse model demonstrates that treatment with a sclerostin-neutralizing antibody (SclAb), which targets an antagonist of canonical WNT\/\u03b2-catenin signaling pathway, fully rescues the low bone mass phenotype to wild-type levels. Together, this study reveals that the \u03b2-catenin signaling cascade is a therapeutic target for restoring defective skeletal microarchitecture in HGPS. \u00a9 2018 American Society for Bone and Mineral Research.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jbmr.3549","type":"journal-article","created":{"date-parts":[[2018,7,12]],"date-time":"2018-07-12T17:05:24Z","timestamp":1531415124000},"page":"2059-2070","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Diminished Canonical \u03b2-Catenin Signaling During Osteoblast Differentiation Contributes to Osteopenia in Progeria"],"prefix":"10.1093","volume":"33","author":[{"given":"Ji Young","family":"Choi","sequence":"first","affiliation":[{"name":"Department of Cell Biology and Molecular GeneticsUniversity of MarylandCollege ParkMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jim K","family":"Lai","sequence":"additional","affiliation":[{"name":"Department of OrthopedicsUniversity of Maryland School of MedicineBaltimoreMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zheng-Mei","family":"Xiong","sequence":"additional","affiliation":[{"name":"Department of Cell Biology and Molecular GeneticsUniversity of MarylandCollege ParkMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret","family":"Ren","sequence":"additional","affiliation":[{"name":"Department of Cell Biology and Molecular GeneticsUniversity of MarylandCollege ParkMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megan C","family":"Moorer","sequence":"additional","affiliation":[{"name":"Department of OrthopedicsUniversity of Maryland School of MedicineBaltimoreMDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph P","family":"Stains","sequence":"additional","affiliation":[{"name":"Department of 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